Sweet's syndrome revisited: a review of disease concepts

Abstract

Sweet's syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by a constellation of symptoms and findings: fever, neutrophilia, erythematous and tender skin lesions that typically show an upper dermal infiltrate of mature neutrophils, and prompt improvement of both symptoms and lesions after the initiation of treatment with systemic corticosteroids. Hundreds of patients with this dermatosis have been reported. The manifestations of Sweet's syndrome in these individuals have not only confirmed those originally described by Dr Robert Douglas Sweet in 1964, but have also introduced new features that have expanded the clinical and pathologic concepts of this condition. The history, clinical characteristics, laboratory findings, associated diseases, pathology, and treatment options of Sweet's syndrome are reviewed. The evolving and new concepts of this dermatosis that are discussed include: (i) Sweet's syndrome occurring in the clinical setting of a disease-related malignancy, or medication, or both; (ii) detection of additional sites of extracutaneous Sweet's syndrome manifestations; (iii) discovery of additional Sweet's syndrome-associated diseases; (iv) variability of the composition and/or location of the cutaneous inflammatory infiltrate in Sweet's syndrome lesions; and (v) additional efficacious treatments for Sweet's syndrome.

Síndrome de Sweet: estudo de 23 casos

“…5 Often, the syndrome may be preceded by an infection (of the upper respiratory tract, which is more common, or gastrointestinal tract) or even be associated with inflammatory bowel disease or pregnancy. 3,4,[6][7][8] An association between Sweet's Syndrome and the pathergy phenomenon -hypersensitivity of the skin more common in Behçet's disease and pyoderma gangrenosum -has also been described. 2.3 Extracutaneous manifestations include constitutional symptoms such as fever, malaise, diffuse pain, arthralgia, and myalgia.…”

“…2,3,4,7 In cases associated with malignancy or drug-induced SS, treatment of the underlying disease and discontinuation of the drug, respectively, are associated with improvement of the condition. 2,3,7 Without treatment, SS lesions usually persist for several months, some, however, may have spontaneous resolution.The disease is characterized by frequent recurrences, which occur in both patients who have been treated and patients who had spontaneous remission. 2,3,7 In patients with SS associated with malignancy, relapses are more common and can be an early sign of recurrence of the underlying disease.…”

Sweet's syndrome in prostate cancer

“…Drug-induced SS is a well-recognized entity with granulocyte colony-stimulating factor most commonly responsible. 1 SS has not been associated with any of the medications taken by our patient and, although, case reports have associated SS with antiinflammatories (diclofenac and celecoxib). It seems unlikely that ibuprofen was causative, given the many years of prior exposure.…”

“…Involvement of ocular and oral mucosa, as in the present case, are well reported. 1 Other sites, which may be involved, include central nervous system, musculoskeletal system, kidneys, gastrointestinal tract, liver and lung. Laboratory abnormalities include neutrophilia and elevated erythrocyte sedimentation rate; however, large series have shown that in many cases these are normal.…”

“…2 Biopsy of the rash typically shows a diffuse infiltrate with large numbers of neutrophils diffusely located predominantly in the upper dermis. 1 Sweet's syndrome is associated with underlying malignancy in up to 20% of cases with haematological malignancies predominating, most commonly acute myeloid leukaemia. 2,3 Previous reviews have suggested that solid tumours account for only 15% of malignancyassociated SS.…”